Publish in this journal
Journal Information
Vol. 52. Issue 10.
Pages 535-536 (October 2016)
Vol. 52. Issue 10.
Pages 535-536 (October 2016)
Letter to the Editor
DOI: 10.1016/j.arbr.2016.04.004
Full text access
Autopsy Case of Pulmonary Artery Sarcoma Forming Aneurysm Without FDG Uptake
Autopsia de un sarcoma de arteria pulmonar, que formaba un aneurisma sin captación de FDG
Keisuke Watanabea,b,
Corresponding author

Corresponding author.
, Masaharu Shinkaia, Takeshi Kanekoc
a Respiratory Disease Center, Yokohama City University Medical Center, Yokohama, Japan
b Department of Medicine, McMaster University, Hamilton, Ontario, Canada
c Department of Pulmonology, Yokohama City University Graduate School of Medicine, Yokohama, Japan
Article information
Full Text
Download PDF
Figures (1)
Full Text
To the Editor:

Pulmonary artery (PA) sarcoma is a rare tumor,1 with clinical symptoms and radiological findings that often resemble pulmonary emboli.2 Main PA aneurysm is an uncommon presentation of PA sarcoma. To the best of our knowledge, only 2 cases have been reported,3,4 neither of which was evaluated with 2-deoxy-2-[18F]fluoro-d-glucose (FDG) positron emission tomography (PET)/computed tomography (CT). We report a case of main PA aneurysm due to PA sarcoma without FDG uptake. This is the first autopsy case report of PA sarcoma with main PA aneurysm.

A 38 year-old-man was referred to our hospital for right main PA aneurysm and well-defined multiple nodules in both lungs on chest CT. PET/CT showed FDG uptake in multiple lung nodules, but not in PA aneurysm (Fig. 1A and B). Transthoracic echocardiography and transesophageal echocardiography revealed PA aneurysm compressing left atrium. PA aneurysm due to PA sarcoma with multiple lung metastases was suspected, and the patient underwent thoracoscopic biopsy and pericardial fenestration. PA leiomyosarcoma was diagnosed on the basis of microscopic findings. Surgery was ruled out due to the prognosis and the invasiveness of surgical management. The patient received chemotherapy with pazopanib, which was stopped due to an allergic reaction with rash and fever. He was scheduled for second line chemotherapy, but died suddenly 2 months after the diagnosis. An autopsy was performed with the consent of his family about 26h after death.

Fig. 1.

PET/CT showed FDG uptake in multiple lung nodules (A), but not in PA aneurysm (B). The thin arrow indicates PA aneurysm (B). Macroscopic findings from the specimen obtained by autopsy revealed PA leiomyosarcoma with cystic degeneration (C). PA, pulmonary artery; LA, left atrium; RA, right atrium.


The autopsy revealed main PA leiomyosarcoma with cystic degeneration (Fig. 1C) and multiple lung and myocardial metastasis. A large amount of blood was found in left thoracic cavity. Pathological findings were consistent with PA leiomyosarcoma with ruptured PA aneurysm. It was speculated that the cause of death was rupture of PA aneurysm.

In general, PA sarcoma shows FDG uptake.2 In this case, PET-CT showed FDG accumulation in multiple lung metastases, but not in pulmonary artery aneurysm. This is because the wall of the aneurysm is too thin for FDG-PET to show FDG uptake. Because of false negatives, therefore, it might be difficult to detect primary pulmonary artery sarcoma with FDG-PET in the presence of an aneurysm.

Two out of 3 patients with main PA aneurysm due to PA sarcoma (2 cases3,4 have been reported in addition to the present case) experienced rupture of main PA aneurysm. Rupture of PA aneurysm is rare because of low pressure of PA.5 But PA aneurysm due to PA sarcoma might be fragile and have a higher risk of rupture than main PA aneurysm due to other diseases.

In conclusion, it might be difficult to detect primary lesion of PA sarcoma with FDG-PET in the presence of an aneurysm. More cases are needed to determine the clinical feature of PA sarcoma with main PA aneurysm.


We would like to thank Dr. Yoshiaki Inayama from the Department of Pathology, Yokohama City University Medical Center for pathological advice, and Dr. Zenko Nagashima from the Department of Cardiology, Yokosuka City Hospital for cardiological advice.

S.H. Blackmon, D.C. Rice, A.M. Correa, R. Mehran, J.B. Putnam, W.R. Smythe, et al.
Management of primary pulmonary artery sarcomas.
Ann Thorac Surg, 87 (2009), pp. 977-984
K. Ito, K. Kubota, M. Morooka, Y. Shida, K. Hasuo, H. Endo, et al.
Diagnostic usefulness of 18F-FDG PET/CT in the differentiation of pulmonary artery sarcoma and pulmonary embolism.
Ann Nucl Med, 23 (2009), pp. 671-676
G.A. De La Cerda Belmont, C.A. Lezama Urtecho.
Pleomorphic malignant histiocytoma of pulmonary arteries presenting as pulmonary aneurysms.
Ann Thorac Surg, 95 (2013), pp. 1091-1093
A. Koch, G. Mechtersheimer, U. Tochtermann, M. Karck.
Ruptured pseudoaneurysm of the pulmonary artery – rare manifestation of a primary pulmonary artery sarcoma.
Interact Cardiovasc Thorac Surg, 10 (2010), pp. 120-121
G.R. Veldtman, J.A. Dearani, C.A. Warnes.
Low pressure giant pulmonary artery aneurysms in the adult: natural history and management strategies.
Heart, 89 (2003), pp. 1067-1070

Please cite this article as: Watanabe K, Shinkai M, Kaneko T. Autopsia de un sarcoma de arteria pulmonar, que formaba un aneurisma sin captación de FDG. Arch Bronconeumol. 2016;52:535–536.

Archivos de Bronconeumología (English Edition)

Subscribe to our newsletter

Article options
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

es en
Política de cookies Cookies policy
Utilizamos cookies propias y de terceros para mejorar nuestros servicios y mostrarle publicidad relacionada con sus preferencias mediante el análisis de sus hábitos de navegación. Si continua navegando, consideramos que acepta su uso. Puede cambiar la configuración u obtener más información aquí. To improve our services and products, we use "cookies" (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here.